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Record Information
Version4.0
StatusExpected but not Quantified
Creation Date2005-11-16 15:48:42 UTC
Update Date2018-03-13 21:31:49 UTC
HMDB IDHMDB0004836
Secondary Accession Numbers
  • HMDB04836
Metabolite Identification
Common NameGalabiosylceramide (d18:1/22:0)
DescriptionGalabiosylceramide is a non-acidic diglycosphingolipids, i.e. a sphingolipid with two or more carbohydrate moieties attached to a ceramide unit. It is a vital component of cellular membranes of most eukaryotic organisms and some bacteria. Its abundance relative to other lipids is usually low other than in epithelial and neuronal cells. Galabiosylceramide has also been found in small amounts in kidney and pancreas, for example. Galabiosylceramide is one of the lipids that accumulates in excessive amounts in Fabry's disease. It is the precursor of the gala series of oligoglycosylceramides. An animal tissues, biosynthesis involves addition of a second monosaccharides unit from the appropriate sugar nucleotide to a monoglycosylceramide, catalysed by a glycosyl transferase, in the lumen of the Golgi apparatus. Glycolipids are important components of the body's immune defense system, either in haptenic reactivity or in antibody-producing potency, i.e. as cellular immunogens or antigens. Certain glycolipids are involved in the antigenicity of blood group determinants, while others bind to specific toxins or bacteria. Some also function as receptors for cellular recognition, and they can be specific for particular tissues or tumours.
Structure
Thumb
Synonyms
ValueSource
1-O-(4-O-alpha-D-Galactopyranosyl-beta-D-galactopyranosyl)-ceramideHMDB
1-O-(4-O-alpha-delta-Galactopyranosyl-beta-delta-galactopyranosyl)-ceramideHMDB
DigalactosylceramideHMDB
Gal-alpha1->4gal-beta1->1'cerHMDB
Chemical FormulaC52H99NO13
Average Molecular Weight946.3414
Monoisotopic Molecular Weight945.711642259
IUPAC NameN-[(4Z)-1-{[(2R,3R,4R,5R,6R)-3,4-dihydroxy-6-(hydroxymethyl)-5-{[(2R,3R,4S,5R,6R)-3,4,5-trihydroxy-6-(hydroxymethyl)oxan-2-yl]oxy}oxan-2-yl]oxy}-3-hydroxyoctadec-4-en-2-yl]docosanamide
Traditional NameN-[(4Z)-1-{[(2R,3R,4R,5R,6R)-3,4-dihydroxy-6-(hydroxymethyl)-5-{[(2R,3R,4S,5R,6R)-3,4,5-trihydroxy-6-(hydroxymethyl)oxan-2-yl]oxy}oxan-2-yl]oxy}-3-hydroxyoctadec-4-en-2-yl]docosanamide
CAS Registry NumberNot Available
SMILES
CCCCCCCCCCCCCCCCCCCCCC(=O)NC(CO[C@@H]1O[C@H](CO)[C@H](O[C@H]2O[C@H](CO)[C@H](O)[C@H](O)[C@H]2O)[C@H](O)[C@H]1O)C(O)\C=C/CCCCCCCCCCCCC
InChI Identifier
InChI=1S/C52H99NO13/c1-3-5-7-9-11-13-15-17-18-19-20-21-22-24-26-28-30-32-34-36-44(57)53-40(41(56)35-33-31-29-27-25-23-16-14-12-10-8-6-4-2)39-63-51-49(62)47(60)50(43(38-55)65-51)66-52-48(61)46(59)45(58)42(37-54)64-52/h33,35,40-43,45-52,54-56,58-62H,3-32,34,36-39H2,1-2H3,(H,53,57)/b35-33-/t40?,41?,42-,43-,45+,46+,47-,48-,49-,50+,51-,52-/m1/s1
InChI KeyQYWVASPEUXEHSY-XXHPVCCSSA-N
Chemical Taxonomy
DescriptionThis compound belongs to the class of organic compounds known as glycosyl-n-acylsphingosines. These are compounds containing a sphingosine linked to a simple glucosyl moiety.
KingdomOrganic compounds
Super ClassLipids and lipid-like molecules
ClassSphingolipids
Sub ClassGlycosphingolipids
Direct ParentGlycosyl-N-acylsphingosines
Alternative Parents
Substituents
  • Glycosyl-n-acylsphingosine
  • Fatty acyl glycoside
  • Fatty acyl glycoside of mono- or disaccharide
  • Alkyl glycoside
  • Disaccharide
  • Glycosyl compound
  • O-glycosyl compound
  • Fatty amide
  • N-acyl-amine
  • Oxane
  • Fatty acyl
  • Secondary carboxylic acid amide
  • Secondary alcohol
  • Carboxamide group
  • Organoheterocyclic compound
  • Polyol
  • Oxacycle
  • Carboxylic acid derivative
  • Acetal
  • Organic nitrogen compound
  • Hydrocarbon derivative
  • Organic oxide
  • Organopnictogen compound
  • Organic oxygen compound
  • Alcohol
  • Organonitrogen compound
  • Carbonyl group
  • Organooxygen compound
  • Primary alcohol
  • Aliphatic heteromonocyclic compound
Molecular FrameworkAliphatic heteromonocyclic compounds
External DescriptorsNot Available
Ontology
Physiological effect

Organoleptic effect:

Disposition

Route of exposure:

Source:

Biological location:

Process

Naturally occurring process:

Role

Industrial application:

Biological role:

Physical Properties
StateSolid
Experimental Properties
PropertyValueReference
Melting PointNot AvailableNot Available
Boiling PointNot AvailableNot Available
Water Solubility0Not Available
LogPNot AvailableNot Available
Predicted Properties
PropertyValueSource
Water Solubility0.0032 g/LALOGPS
logP7.05ALOGPS
logP9.99ChemAxon
logS-5.5ALOGPS
pKa (Strongest Acidic)11.92ChemAxon
pKa (Strongest Basic)-3ChemAxon
Physiological Charge0ChemAxon
Hydrogen Acceptor Count13ChemAxon
Hydrogen Donor Count9ChemAxon
Polar Surface Area227.86 ŲChemAxon
Rotatable Bond Count42ChemAxon
Refractivity258.21 m³·mol⁻¹ChemAxon
Polarizability115.27 ųChemAxon
Number of Rings2ChemAxon
Bioavailability0ChemAxon
Rule of FiveYesChemAxon
Ghose FilterYesChemAxon
Veber's RuleYesChemAxon
MDDR-like RuleYesChemAxon
Spectra
SpectraNot Available
Biological Properties
Cellular Locations
  • Cytoplasm
  • Extracellular
  • Membrane
  • Lysosome
  • Golgi apparatus
Biospecimen LocationsNot Available
Tissue Location
  • Brain
  • Heart
  • Kidney
Pathways
NameSMPDB/PathwhizKEGG
Fabry diseaseThumbThumb?image type=greyscaleThumb?image type=simpleNot Available
Gaucher DiseaseThumbThumb?image type=greyscaleThumb?image type=simpleNot Available
Globoid Cell LeukodystrophyThumbThumb?image type=greyscaleThumb?image type=simpleNot Available
Krabbe diseaseThumbThumb?image type=greyscaleThumb?image type=simpleNot Available
Metachromatic Leukodystrophy (MLD)ThumbThumb?image type=greyscaleThumb?image type=simpleNot Available
Normal Concentrations
Not Available
Abnormal Concentrations
Not Available
Associated Disorders and Diseases
Disease ReferencesNone
Associated OMIM IDsNone
DrugBank IDNot Available
Phenol Explorer Compound IDNot Available
FoodDB IDFDB023437
KNApSAcK IDNot Available
Chemspider ID16744853
KEGG Compound IDC06126
BioCyc IDNot Available
BiGG ID2267787
Wikipedia LinkNot Available
METLIN ID7095
PubChem Compound20057277
PDB IDNot Available
ChEBI IDNot Available
References
Synthesis ReferenceNot Available
Material Safety Data Sheet (MSDS)Not Available
General References
  1. Ohdoi C, Nyhan WL, Kuhara T: Chemical diagnosis of Lesch-Nyhan syndrome using gas chromatography-mass spectrometry detection. J Chromatogr B Analyt Technol Biomed Life Sci. 2003 Jul 15;792(1):123-30. [PubMed:12829005 ]
  2. Falk P, Hoskins LC, Larson G: Enhancing effects of bile salts on the degradation of glycosphingolipids by glycosidases from bacteria of the human fecal flora. Biochim Biophys Acta. 1991 Jul 9;1084(2):139-48. [PubMed:1854798 ]
  3. Ghislain J, Lingwood CA, Fish EN: Evidence for glycosphingolipid modification of the type 1 IFN receptor. J Immunol. 1994 Oct 15;153(8):3655-63. [PubMed:7930586 ]
  4. Burger KN, van der Bijl P, van Meer G: Topology of sphingolipid galactosyltransferases in ER and Golgi: transbilayer movement of monohexosyl sphingolipids is required for higher glycosphingolipid biosynthesis. J Cell Biol. 1996 Apr;133(1):15-28. [PubMed:8601603 ]
  5. van der Bijl P, Lopes-Cardozo M, van Meer G: Sorting of newly synthesized galactosphingolipids to the two surface domains of epithelial cells. J Cell Biol. 1996 Mar;132(5):813-21. [PubMed:8603914 ]
  6. Lanne B, Jondal M, Karlsson KA: Gal alpha 4Gal-binding antibodies: specificity and use for the mapping of glycolipids of Burkitt lymphoma and other human tumors. Glycobiology. 1996 Jun;6(4):423-31. [PubMed:8842706 ]
  7. Ledvinova J, Poupetova H, Hanackova A, Pisacka M, Elleder M: Blood group B glycosphingolipids in alpha-galactosidase deficiency (Fabry disease): influence of secretor status. Biochim Biophys Acta. 1997 Apr 1;1345(2):180-7. [PubMed:9106497 ]

Only showing the first 10 proteins. There are 61 proteins in total.

Enzymes

General function:
Involved in galactosylceramide sulfotransferase activity
Specific function:
Catalyzes the sulfation of membrane glycolipids. Seems to prefer beta-glycosides at the non-reducing termini of sugar chains attached to a lipid moiety. Catalyzes the synthesis of galactosylceramide sulfate (sulfatide), a major lipid component of the myelin sheath and of monogalactosylalkylacylglycerol sulfate (seminolipid), present in spermatocytes (By similarity). Also acts on lactosylceramide, galactosyl 1-alkyl-2-sn-glycerol and galactosyl diacylglycerol (in vitro).
Gene Name:
GAL3ST1
Uniprot ID:
Q99999
Molecular weight:
48763.63
General function:
Involved in catalytic activity
Specific function:
Not Available
Gene Name:
GLA
Uniprot ID:
P06280
Molecular weight:
Not Available
General function:
Involved in cysteine-type endopeptidase activity
Specific function:
Mediates GPI anchoring in the endoplasmic reticulum, by replacing a protein's C-terminal GPI attachment signal peptide with a pre-assembled GPI. During this transamidation reaction, the GPI transamidase forms a carbonyl intermediate with the substrate protein
Gene Name:
PIGK
Uniprot ID:
Q92643
Molecular weight:
45251.4
General function:
Involved in biosynthetic process
Specific function:
Necessary for the synthesis of N-acetylglucosaminyl-phosphatidylinositol, the very early intermediate in GPI-anchor biosynthesis.
Gene Name:
PIGA
Uniprot ID:
P37287
Molecular weight:
54126.065
General function:
Involved in catalytic activity
Specific function:
Non-lysosomal glucosylceramidase that catalyzes the conversion of glucosylceramide to free glucose and ceramide. Involved in sphingomyelin generation and prevention of glycolipid accumulation. May also catalyze the hydrolysis of bile acid 3-O-glucosides, however, the relevance of such activity is unclear in vivo.
Gene Name:
GBA2
Uniprot ID:
Q9HCG7
Molecular weight:
104648.13
General function:
Involved in GPI anchor biosynthetic process
Specific function:
Component of the GPI transamidase complex. May be involved in the recognition of either the GPI attachment signal or the lipid portion of GPI
Gene Name:
PIGU
Uniprot ID:
Q9H490
Molecular weight:
50051.2
General function:
Involved in protein binding
Specific function:
Component of the GPI transamidase complex. Essential for transfer of GPI to proteins, particularly for formation of carbonyl intermediates
Gene Name:
PIGT
Uniprot ID:
Q969N2
Molecular weight:
65699.0
General function:
Involved in transferase activity, transferring hexosyl groups
Specific function:
Catalyzes the formation of some glycolipid via the addition of N-acetylgalactosamine (GalNAc) in alpha-1,3-linkage to some substrate. Glycolipids probably serve for adherence of some pathogens
Gene Name:
GBGT1
Uniprot ID:
Q8N5D6
Molecular weight:
40126.9
General function:
Involved in transferase activity, transferring hexosyl groups
Specific function:
Mannosyltransferase involved in glycosylphosphatidylinositol-anchor biosynthesis. Transfers the first alpha-1,4-mannose to GlcN-acyl-PI during GPI precursor assembly
Gene Name:
PIGM
Uniprot ID:
Q9H3S5
Molecular weight:
49459.2
General function:
Involved in protein binding
Specific function:
Component of the GPI transamidase complex. Essential for transfer of GPI to proteins, particularly for formation of carbonyl intermediates
Gene Name:
PIGS
Uniprot ID:
Q96S52
Molecular weight:
61655.5

Only showing the first 10 proteins. There are 61 proteins in total.